Pediatric blood & cancer
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Pediatric blood & cancer · Mar 2010
Multicenter StudyTrilateral retinoblastoma: potentially curable with intensive chemotherapy.
Trilateral retinoblastoma has been lethal in virtually all cases previously reported. We describe a series of 13 patients treated with intensive chemotherapy, defined as the intention to include high-dose chemotherapy with autologous hematopoietic stem cell rescue. ⋯ Intensive chemotherapy is potentially curative for some patients with trilateral retinoblastoma, especially those with M-0 disease.
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Pediatric blood & cancer · Mar 2010
Functional independence score in hemophilia: a cross-sectional study assessment of Mexican children.
The Functional Independence Score in Hemophilia (FISH) is a performance-based assessment tool used to measure the patients functional ability so far only used in patients with severe hemophilia. Its aim is to determine if FISH is useful in patients with mild and moderate disease. ⋯ A significant decrease in functional ability was demonstrated according to the severity of hemophilia, especially for those activities involving weight-bearing demands like locomotion and step climbing. Of seven activities evaluated, changes were observed in the group with moderate and severe hemophilia, but no changes detected in patients with mild disease.
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Pediatric blood & cancer · Mar 2010
Pre-transplant lung function is predictive of survival following pediatric bone marrow transplantation.
Pulmonary toxicity is well described in recipients of bone marrow transplants (BMT), and accounts for a sizeable proportion of post-transplant mortality. The majority of the data on post-transplant pulmonary function is from adults, although several small pediatric case series have been described. In adults, pre-transplant lung function has been predictive of post-transplant respiratory failure and mortality. This use of pulmonary function testing, that is, for pre-transplant risk counseling, is novel but has never been applied to pediatric patients. We hypothesized that in children, as in adults, pre-transplant pulmonary function would also be predictive of outcome post-transplantation morbidity. ⋯ Lung function prior to bone marrow transplant, especially diffusing capacity, is frequently abnormal. Lung function frequently decreases shortly post-transplant and tends to improve over time, but frequently remains abnormal even years after transplant. Post-transplant survival is related to pre-transplant lung function.
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Pediatric blood & cancer · Mar 2010
Pulmonary hypertension in children and young adults with sickle cell disease: evidence for familial clustering.
Pulmonary hypertension (PHTN) is increasingly recognized as a serious complication of sickle cell disease (SCD). Our objective was to determine the prevalence of PHTN and identify factors associated with PHTN among children and young adults with SCD in Lebanon. ⋯ PHTN was common, associated with increased hemolysis but not G6PD deficiency, and clustered in families. Moreover, PHTN developed despite hydroxyurea therapy in five patients.